Submitted by: Ma. Kay Bernadette T.

Lumbab, RN

Brugada Syndrome
Definition Brugada syndrome is a potentially life-threatening heart rhythm disorder. t!s "hara"teri#ed by a spe"ifi" abnormal heartbeat, dete"ted by an ele"tro"ardiogram test, "alled a Brugada sign. Brugada syndrome is fre$uently an inherited "ondition. Many people %ho ha&e Brugada syndrome don!t ha&e any symptoms, and so they!re una%are of their "ondition. 'or some, ho%e&er, Brugada syndrome "an "ause dangerous irregular heart rhythms that "an "ause fainting or sudden "ardia" arrest. Brugada syndrome is treatable using an implanted medi"al de&i"e "alled an implantable "ardio&erterdefibrillator. Be"ause Brugada syndrome %as dis"o&ered re"ently, resear"h is still under %ay on other treatments for the disorder. Symptoms Many people %ho ha&e Brugada syndrome are undiagnosed be"ause the "ondition often hasn!t "aused any noti"eable signs or symptoms. The most important sign or symptom of Brugada syndrome is an abnormal heart rhythm (arrhythmia) "alled a Brugada sign. * Brugada sign is a pattern of heartbeats that!s found on a test of your heart rhythm (ele"tro"ardiogram, or +,-). .ou "an!t feel a Brugada sign / it!s only dete"ted on an +,-. t!s possible to ha&e a Brugada sign %ithout ha&ing Brugada syndrome. 0o%e&er, signs and symptoms that "ould mean you ha&e Brugada syndrome in"lude:

'ainting (syn"ope) rregular heartbeats or palpitations Stopped heartbeat (sudden "ardia" arrest)

Brugada syndrome signs and symptoms are similar to some other heart rhythm problems, so it!s essential that you see your do"tor to find out if Brugada syndrome or another heart rhythm problem is "ausing your symptoms. When to see a doctor 1. f you ha&e heart palpitations or an irregular heartbeat (arrhythmia), ma2e an appointment to see your do"tor. .our problem "ould be "aused by a heart rhythm problem, but tests %ill be ne"essary to see if your heart problem is Brugada syndrome. f you faint and you suspe"t it may be be"ause of a heart "ondition, see2 emergen"y medi"al attention. 3. f your parent, sibling or "hild has been diagnosed %ith Brugada syndrome, you may also %ant to ma2e an appointment %ith your do"tor. 0e or she "an dis"uss %hether you should undergo geneti" testing to see if you!re at ris2 of Brugada syndrome. Causes Brugada syndrome is a heart rhythm disorder. +a"h beat of your heart is triggered by an ele"tri"al impulse generated by spe"ial "ells in the right upper "hamber of your heart. Tiny pores, "alled "hannels, on ea"h of these "ells dire"t this ele"tri"al a"ti&ity, %hi"h ma2es your heart beat. n Brugada syndrome, a defe"t in these "hannels "an "ause your heart to beat abnormally. 4uring these episodes, your heart doesn!t pump effe"ti&ely. *s a result, not enough blood tra&els to the rest of your body. This "an "ause fainting, other heart rhythm disorders, or in e5treme "ases, sudden "ardia" death.

Brugada syndrome is usually inherited, but it may also result from a stru"tural abnormality in your heart, imbalan"es in "hemi"als that help transmit ele"tri"al signals through your body (ele"trolytes), or the effe"ts of "ertain pres"ription medi"ations or "o"aine use. Brugada syndrome is usually diagnosed in adoles"ents and adults. t!s rarely diagnosed in young "hildren. Risk factors Risk factors for Brugada syndrome include:

Family history of Brugada syndrome. f other family members ha&e had Brugada syndrome, you!re at an in"reased ris2 of ha&ing the "ondition. Being male. *dult men are more fre$uently diagnosed than are %omen. n young "hildren and adoles"ents, ho%e&er, boys and girls are diagnosed at about the same rate. Race. Brugada syndrome o""urs more fre$uently in *sians than in other ra"es. Fever. 6hile ha&ing a fe&er doesn!t bring on Brugada syndrome itself, fe&er "an in"rease the ris2 of fainting or other "ompli"ations of Brugada syndrome, espe"ially in "hildren.

Complications The most serious "ompli"ation of Brugada syndrome is sudden "ardia" arrest. Sudden "ardia" arrest is the sudden, une5pe"ted loss of heart fun"tion, breathing and "ons"iousness. t!s a medi"al emergen"y. f not treated immediately, the "ondition is fatal, resulting in sudden "ardia" death. 6ith fast, appropriate medi"al "are, sur&i&al is possible. *dministering "ardiopulmonary resus"itation (,7R) / or e&en 8ust rapid "ompressions to the "hest / "an impro&e the "han"es of sur&i&al until emergen"y personnel arri&e. Sudden fainting (syn"ope) is another "ompli"ation of Brugada syndrome. f you ha&e Brugada syndrome and you faint, see2 emergen"y medi"al attention. Preparing for your appointment f your do"tor thin2s you ha&e Brugada syndrome, you!ll li2ely need se&eral appointments to ma2e sure that!s the "ondition you ha&e, and to figure out ho% serious your "ondition is. .our do"tor should gi&e you instru"tions before ea"h appointment on spe"ifi" preparations. Be"ause appointments "an be brief, and be"ause there!s often a lot of ground to "o&er, it!s a good idea to be prepared for your appointment. 0ere!s some information to help you get ready for your appointment, and %hat to e5pe"t from your do"tor. What you can do

Be aware of any pre appointment restrictions. *t the time you ma2e the appointment, be sure to as2 if there!s anything you need to do in ad&an"e. 'or e5ample, if you!re ha&ing a test to loo2 at your heart!s stru"ture (ele"trophysiology study), you!ll need to fast for eight to 13 hours before your test. Write down any symptoms you!re e"periencing# in"luding any that may seem unrelated to Brugada syndrome. Write down key personal information# espe"ially any family history of sudden "ardia" arrest or heart "onditions, and any personal history of fainting spells or heart arrhythmias. $ake a list of all medications# as %ell as any &itamins or supplements, that you!re ta2ing. %ake a family mem&er or friend along# if possible. Sometimes it "an be diffi"ult to soa2 up all the information pro&ided to you during an appointment. Someone %ho a""ompanies you may remember something that you missed or forgot. Write down 'uestions to ask your do"tor.

.our time %ith your do"tor is limited, so preparing a list of $uestions %ill help you ma2e the most of your time together. List your $uestions from most important to least important in "ase time runs out. 'or Brugada syndrome, some basi" $uestions to as2 your do"tor in"lude:

6hat is li2ely "ausing my symptoms or "ondition9 6hat are other possible "auses for my symptoms or "ondition9 6hat 2inds of tests %ill need9

6ill need treatment for Brugada syndrome9 6hat is the best "ourse of a"tion9 6hat!s an appropriate le&el of physi"al a"ti&ity9 0o% often do need follo%-up &isits to monitor my "ondition9 ha&e other health "onditions. 0o% "an best manage them together9 *re there any restri"tions that need to follo%9 Should see a spe"ialist9 *re there any bro"hures or other printed material that "an ta2e home %ith me9 6hat 6eb sites do you re"ommend &isiting9 Should any members of my family be s"reened9 n addition to the $uestions that you!&e prepared to as2 your do"tor, don!t hesitate to as2 $uestions during your appointment at any time that you don!t understand something.

What to e"pect from your doctor .our do"tor is li2ely to as2 you a number of $uestions. Being ready to ans%er them may reser&e time to go o&er any points you %ant to spend more time on. .our do"tor may as2:

4o you ha&e a family history of Brugada syndrome or other heart rhythm problems9 6hen did you first begin e5perien"ing symptoms9 0a&e your symptoms been "ontinuous or o""asional9 0o% often do you ha&e symptoms, su"h as fainting spells9

%ests and diagnosis *side from a typi"al physi"al e5amination and listening to your heart %ith a stethos"ope, tests to see if you ha&e Brugada syndrome in"lude:

(lectrocardiogram )(C*+ with medication. n this nonin&asi&e test, a te"hni"ian %ill pla"e probes on your "hest that re"ord the ele"tri"al impulses that ma2e your heart beat. *n +,- re"ords these ele"tri"al signals and "an help your do"tor dete"t irregularities in your heart!s rhythm and stru"ture. 0o%e&er, be"ause your heart rhythm "an "hange, an ele"tro"ardiogram by itself may not dete"t an abnormal heart rhythm. .our do"tor may gi&e you a medi"ation that "auses an abnormal heart rhythm in people %ho ha&e Brugada syndrome. The medi"ation is usually in8e"ted by an intra&enous ( :) line.

(lectrophysiology )(P+ test. f your +,- suggests that you ha&e Brugada syndrome, your do"tor may also re"ommend an +7 test to pinpoint %here in your heart your arrhythmia o""urs and to "he"2 the se&erity of your "ondition. n an +7 test, a "atheter is threaded through a &ein in your groin to your heart, similar to "ardia" "atheteri#ation. +le"trodes are then passed through the "atheter to different points in your heart. The ele"trodes then map out any irregular heartbeats. The ele"trodes don!t sho"2 your heart / they 8ust dete"t the ele"tri"al signals running through your heart.

*enetic testing. 6hile geneti" testing isn!t re$uired to diagnose Brugada syndrome, your do"tor may re"ommend geneti" testing for other family members if you!re diagnosed %ith Brugada syndrome. The a&ailable geneti" tests for Brugada are reliable.

%reatments and drugs Treatment of Brugada syndrome depends on the ris2 of an abnormal heartbeat (arrhythmia). Those "onsidered at high ris2 ha&e:

* family history of sudden "ardia" death * personal history of serious heart rhythm problems * personal history of se&ere fainting spells

Be"ause of the nature of the heart rhythm abnormality, medi"ations "an!t treat Brugada syndrome / only a medi"al de&i"e "alled an implantable "ardio&erter-defibrillator "an. mplanting the de&i"e is usually re"ommended for people at high ris2 of sudden "ardia" death or other "ompli"ations of Brugada syndrome.

,mplanta&le cardioverter defi&rillator ),CD+. 'or high-ris2 indi&iduals, treatment may in"lude an implantable "ardio&erter-defibrillator ( ,4). This small de&i"e "ontinuously monitors your heart rhythm and deli&ers ele"tri"al sho"2s %hen needed to "ontrol abnormal heartbeats. The pro"edure to implant an ,4 re$uires hospitali#ation for a day or t%o. There!s a ris2 of "ompli"ations from ha&ing an ,4 implanted. 7eople %ho ha&e an ,4 implanted as a treatment for Brugada syndrome ha&e reported re"ei&ing sho"2s from their ,4 e&en %hen their heartbeat %as regular. This may be be"ause many people %ho re"ei&e an ,4 as a treatment for Brugada syndrome are young, and may re"ei&e sho"2s %hen their heart rates in"rease during normal stresses, su"h as e5er"ise. .our do"tor %ill program your ,4 to redu"e this ris2. f you ha&e an ,4 implanted as part of your Brugada syndrome treatment, tal2 to your do"tor about %ays to a&oid inappropriate sho"2s.

Coping and support 'inding out you ha&e Brugada syndrome "an be diffi"ult. .ou may %orry if your treatment %ill %or2 or if other family members "ould be at ris2. There are %ays to "ope %ith your feelings about your "ondition, in"luding:

Support groups. 'inding out that you or a lo&ed one has heart disease "an be unner&ing. Turning to friends and family for support is essential, but if you find you need more help, tal2 to your do"tor about 8oining a support group. .ou may find that tal2ing about your "on"erns %ith others %ho are e5perien"ing the same diffi"ulties "an help. Continued medical checkups. f you ha&e Brugada syndrome, it!s a good idea to regularly "he"2 in %ith your do"tor to ma2e sure you!re properly managing your heart "ondition. Regular "he"2ups "an help your do"tor de"ide if you need to "hange your treatment, and may help "at"h ne% problems early, if they o""ur.